
Objectives: Although only three studies are available in Saudi Arabia concerning celiac disease (CD) in Down Syndrome (DS), they showed considerable variation in the serological prevalence (4-15.5 %) and in the prevalence of biopsy-proven CD (2-10.7%). Thus, we aim to use meta-analysis to examine the prevalence of celiac disease (CD) in patients with down syndrome (DS) in Saudi Arabia (SA) using meta-analysis for these three studies. Methods: We used the comprehensive systematic search through database and journals followed by selection processes. For data analysis we used two programs: 1-The statistical package for social science (IBM SPSS Inc). 2- The Comprehensive Meta-analysis program (CMA). Results: The three related articles involved 226 (51-91) DS patients with age range (0.5-18 years) covering children and adolescents. The CD positive patients showed considerable variation (4-15.5%) for the serological prevalence and (2-10.7%) for the prevalence of biopsy-proven CD. By Meta analysis the seroprevalence of CD (one serology at least) was 13.4% with moderate heterogeneity (I2=46.519), while the prevalence of biopsy-proven CD 9.4% with moderate heterogeneity (I2=29.229). Anti-tTG was used in all studies; as single in one study; with EMA and AGA in one studies; with EMA and AGA and ARA in one study. Conclusion: People who have DS in SA tend to develop CD at rates (9.4%) higher than the global prevalence (5.8%) and far above those in the Saudi general population (1.4%). The prevalence is high enough to motivate screening CD in DS children. There is a significant difference between the reported serologically- proven rates and the reported biopsy- proven rates (p = 0.002).